I'm Aware That I'm Rare: the phaware® podcast

A new podcast series devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. New Episodes every Monday & Thursday!

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In 2000, Liz DeVivo was diagnosed with Scleroderma, a rare
and chronic autoimmune disease. The disease attacked
her internal organs, causing pulmonary hypertension. Due to
the urgency of her condition, she was placed at the top of the
transplant list in September 2001. Liz wrote a book (Holding On For Dear
Life), chronicling her journey as a young mother to a
heart and double lung transplant. Her transplant story was
also featured in a commercial in
the Super Bowl 51 broadcast.

My name is Liz DeVivo, and I live in Upstate New York in
Binghamton, New York.

I first started having changes to my body in 1996, when I was
pregnant with my second child. The changes came kind of slowly, and
[my doctors] noticed that I had an elevated antinuclear antibody
level, an ANA level, and they thought that that would go away after
I had my baby.

After I had my baby, it did not go away, and then other changes
started to happen. I started to have Raynaud's phenomenon, and I
started to have changes to my hands and face where my skin started
to harden and to thin and to kind of become shinier. My hands were
swelling and my joints started to get arthritic. Those were the
first changes I noticed.

It probably took about two years before they were able to
diagnosis it as scleroderma, which is an autoimmune disease much
like lupus or MS or those types of more familiar autoimmune
diseases. Scleroderma actually means hardening of skin. What
happens is your body overproduces collagen. It can happen to your
skin outside of your body, and it can happen inside your body to
your organs.

When I was first diagnosed, it was really looking like it was
mostly skin involved outside, and so I didn't really pay much
attention to it. I felt okay. I had some fatigue, but I was okay. I
had two little kids. I was going to college. I was tired. I just
assumed that it was because I was a full-time grad student and
taking care of two kids that I was exhausted and kind of out of
shape.

I found myself getting more and more out of breath, and I really
chocked it up to inertia and lack of doing things. I did two weeks
of exercise and realized I wasn't getting any exercise tolerance. I
was actually getting worse. I knew I was in a bit of trouble then…
that something was going on.

I ended up going to the doctor, and my blood pressure was really
high. They decided to send me to the hospital, and when they did
send me to the hospital I had a pericardial effusion, which is
fluid around your heart that builds up in the sac of your heart.
That was the first thing that happened that kind of threw me into
crisis mode, and I knew I wasn't well and something was wrong.

After that, they really thought that was a scleroderma-based
event, and they thought I would heal and go on, but I just didn't
heal and I couldn't get my breath back. I was really struggling
with walking, even just a few yards or a few feet. It was really
hard. Actually, I kept thinking that I would get better and I would
go back to work and I would be fine. It just wouldn't turn
around.

Finally they sent me to a cardiologist, and when I went to the
cardiologist he said, "I'm sorry. You have pulmonary hypertension.
Do you know what that is?" I did know because I knew that that was
a complication for some people with scleroderma. He just looked at
me and he said, "I'm sorry." I said, "Well, what's my prognosis?"
He said, "Maybe two to five years," and that was literally the end
of the discussion. That was all the discussion there was at the
time, and that was the year 2000.

I was devastated after talking to him. I was angry after talking
to him. I was sad after talking to him. But mostly I was just
determined to figure out something. I just couldn't believe that
that was just it.

The first thing I did was go to New York City and find a
pulmonary hypertension specialist in New York. At that time there
was very limited help. There was very limited help on the computer.
There weren't very many support groups. There was very limited
stuff going on. You could find a tiny bit, but not much. I went to
New York hoping someone could give me some kind of hope. I met with
a pulmonary hypertension specialist there, and he did all the
preliminary stuff, the heart catheterization, the echos, and
everything that goes along with that.

Basically, my pulmonary pressures were really high and I was a
pretty good wreck. I think it was in the 120s, when I was first
diagnosed and I could barely walk. I was blue. My lips were blue,
my fingers were blue, my feet were blue. I was pretty blue.

That was in July of 2000, and by September of 2000 I was put on
IV medication, and they thought that perhaps they could add
something to stop the fibrosis or the hardening of my lungs, so I
also did six months of chemotherapy to help stop the hardening of
my lungs. All through that time, I still had the pericardial
effusions, so my heart kept filling up and I kept having to have
... I'd like get to the point of almost cardiac tamponade. Then
they would drain it.

After the third time it happened, they finally decided to just
open up my pericardial sac and leave it open, which was helpful.
That was definitely helpful. That first year that I was diagnosed
was really hard. I was hospitalized over and over and over. It was
like plugging a dike. Every time you'd plug the dike, it would
spring a leak somewhere else, something else would go wrong. I knew
I was running out of time fast. The writing was on the wall pretty
clear. I knew I needed to start looking for other solutions.

I went to a hospital in New York and met with the surgeon there,
and he promptly turned me down right there on the spot, right to my
face, and told me I was too sick and I was a waste of resources, is
how he put it. He didn't think I could survive the illness, and
because it was scleroderma, and scleroderma is not even curable
with a transplant, he couldn't justify the use, I guess. So I was
pretty devastated, completely devastated, but very determined
still.

I had a lot of support from my family and friends, and we just
set about calling and finding out what centers in the U.S. would
even consider me. At that time there were only like three places
that would consider me in Texas and in California, and in
Pittsburgh. Because Pittsburgh is the closest, I started with them.
I called them and made the appointment thinking maybe I needed a
lung or two, and they said, "No, you need a heart and two lungs." I
knew that that was just going to be a harder surgery physically and
a harder surgery to wait for. Those are very rare surgeries with
very few people on the list. I knew my chances were diminishing
fast, and at the time I was only about 95 pounds.

I knew I was waiting for a very small donor, because you can put
small organs in a big body, but you can't put big organs in a small
body. I was waiting for a small person, and I was a B- blood type,
which is pretty rare, so I knew that my odds were not really very
good at all. I waited 15 months before I got my first call for a
transplant. I was in New York City for an educational forum on
scleroderma, and it was November, and who would figure, but it was
actually snowy and icy and windy, and I couldn't get a plane to fly
me to Pittsburgh. I had to turn down the organs, which was
horrible, but I had to believe that they were for someone else.
They just had to be for someone else.

Then I continued my wait, and at 23 months, I finally got the
call, the real deal call. It was in July, July 29th of 2003, and it
was a beautiful sunny day. I went to Pittsburgh and I was not even
afraid at that point because I knew it was my only chance to live.
My surgery itself went pretty well. It was pretty standard, I
think, about maybe eight hours. My surgeon came out and told my
family that everything went well, and now they were just going to
work on closing me up and he would be out in a few hours to let
them know. I guess the few hours turned into many hours and turned
into more hours. I had some problems with bleeding, and they
couldn't stop my heart from bleeding, and they couldn't get my
heart in sync either. I have what's called a clamshell incision,
which goes from my armpit to my armpit, and they literally work on
me like that, like open. This part is really hard for me to really
even fathom or understand.

They worked on me for a week while I was open like that, which
was just crazy to even imagine. I was in a medically-induced coma
for at least a few weeks, if not more, before I was conscious. Even
when I was conscious I had no idea what was going on. I was
hallucinating like crazy. I was in the ICU for like seven weeks. I
just had a really hard time.

Finally, when I got out of surgery and finally when I got out of
the ICU, it probably took another couple months or so before I
actually made it home. I had my transplant in July and I got home
the day before Thanksgiving. It was a pretty long road. It was not
easy. Even at that point, I still didn't even know I was better.
Everyone would say, "Liz, you're pink. Your lips are pink. You look
good," and I couldn't even tell that I was better. It took like six
months after my surgery, and we were walking and I remember
thinking, "My God, I think that wasn't so hard." It was like the
light bulb went off in my head and I couldn't even believe it. "I
think I'm better."

From that moment on the joy just started rushing in and I just
started to heal faster and faster, and feel better and be better.
It was a pretty amazing journey for sure. I think I took about a
whole year to really recoup from that surgery, for sure. My kids
were pretty little at that point. They were 7 and 10, so I was
pretty happy to be their mother and to take care of the, and to
take care of my family, and honored to do those things. My kids
graduated from high school. I returned to work part-time as a
social worker, and I worked in dialysis for about 4-5 years doing
that, which was wonderful.

I spend my time volunteering for Donate Life events, and
educating the public about organ donation, and I also spend time
talking to medical health professionals. I spend a lot of time
talking to nurses, new hires, and fellows and interns when they'll
have me.

I think the most important thing health professionals can give
us is hope. That's like the most important asset, as far as I'm
concerned, that anybody can give somebody who is suffering from a
chronic illness is hope. That's why it's important to share those
stories.

The biggest asset anyone has in their fight to any disease is
their brain, and that's the bottom line. If you believe it can
happen, if it's possible, if you believe it's possible, it can be
possible. If you don't think it's possible because all the data
says it's not possible, it will never be possible.

I started writing a blog while I was sick, and it ended up
becoming a book. My book is called Holding On For Dear
Life: What My Fatal Illness Taught Me About Living. It
started because people kept saying, "Liz, you need to make this a
book. I've learned so much from you. You really need to make this a
book." It was more than just people who are sick even. It was all
kinds of people that were telling me that it had some importance to
them in their life.

I added more to the blog, so it's more than just a blog, and it
became health advice for people going through catastrophic illness,
and it also became like a primer of some kind for healthcare
professionals, too, so it has some advice for healthcare
professionals. It's almost like a How To Survive Your Catastrophic
Illness book. It talks about we're more than just our illness.

We come to the table as patients, and we come with all our
symptoms, but we also come with all our history and our family
members and everything else that comes to the table. We're more
than just the symptoms that you add up in the column. It really is
about hope and the importance of hope in the healing process.

The medicines are changing every minute, every day, every year.
It's amazing the strides that have taken place in the last 10
years, 15 years. It's mind-boggling. When I see the stories of
people who are newly diagnosed, I'm grateful for them because I
know it's so much better than it was. My takeaway is to never
forget that your hope and your brain, it's the greatest asset you
have in fighting this disease.

About the Podcast

A new podcast series devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. New Episodes every Monday & Thursday.
Through this series of impactful, insightful and, most importantly, hopeful stories from members of the global pulmonary hypertension community, we hope to further the global #phaware conversation as well as to capture, engage and enable misdiagnosed and undiagnosed PH patients because early diagnosis and treatment can mean the difference between life and death.
Topics range from the importance of early diagnosis and global PH awareness, to the impact of clinical trials to how patient support and advocacy are paramount to battling this disease.
Learn more about PH at www.phaware.global